RESUMO
BACKGROUND: Gerbode defect is a congenital or acquired communication between the left ventricle and right atrium. While the defect is becoming a more well-recognized complication of cardiac surgery, it presents a diagnostic and therapeutic challenge for providers. This case highlights the predisposing factors and imaging features that may assist in the diagnosis of Gerbode defect, as well as potential approaches to treatment. CASE SUMMARY: We report a patient with severe mitral stenosis as a result of remote mediastinal radiation who underwent extensive decalcification during surgical mitral valve replacement and tricuspid valve repair. Following the procedure, he developed progressive heart failure refractory to medical management. Extensive workup ultimately led to the diagnosis of iatrogenic acquired Gerbode defect. Close collaboration between adult cardiology, cardiothoracic surgery, and the congenital cardiology services led to an optimal treatment plan involving percutaneous closure of the defect. DISCUSSION: Gerbode defect is a rare complication of invasive procedures involving the interventricular septum or its nearby structures. An understanding of the key echocardiographic features will aid providers in timely diagnosis. Percutaneous repair should be strongly considered for patients who may be poor surgical candidates.
RESUMO
BACKGROUND: Left ventricular non-compaction cardiomyopathy (LVNCC) is a rare disease that starts in utero and may progress to heart failure (HF), sometimes requiring orthotopic heart transplantation (OHT). There are limited data addressing characteristics of LVNCC patients that require OHT and their outcomes. We therefore sought to investigate the characteristics and outcomes of LVNCC patients treated with OHT. METHODS: We queried the United Network for Organ Sharing (UNOS) database for all patients listed for OHT with LVNCC as the primary heart failure etiology between 2000 and 2013. We examined their characteristics at listing and outcomes after OHT and compared the findings with those of patients with idiopathic cardiomyopathy (IDCMP). RESULTS: We identified 113 patients (43 adults and 70 pediatrics) with LVNCC of 45,298 patients (0.25% overall, 0.11% of adults and 1.0% of pediatrics) listed for OHT in this time period. Most were male children with mean age at listing of 16.9 years. Compared with the overall IDCMP cohort, patients with LVNCC were younger, had higher use of inotropes and extracorporeal membrane oxygenation (ECMO), and were more often listed as UNOS Status 1A with shorter waiting time. However, when adjusted for age, gender and ethnicity, these differences disappeared. During transplant listing, 8 (7.9%) died, 5 (5.0%) improved and avoided transplant, 3 (3.0%) became too sick for transplant and 78 (77.2%) underwent OHT. There was a non-significant trend toward longer cardiac allograft survival in patients with LVNCC (10.6 vs. 9.4 years; log-rank test, p = 0.068). Patients with LVNCC had similar outcomes to other IDCMP patients, except for more post-transplant infections (50.0% vs. 21.6%, p < 0.05). CONCLUSIONS: LVNCC patients undergoing heart transplantation are mostly pediatric and predominantly bridged to transplant with inotropes or ECMO. Despite having more post-transplant infections, their survival is similar to that of other IDCMP patients.